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Other guidelines for the use of serum free light chain measurement in the management of AL amyloidosis, [42] plasmacytoma [43] and the comparison of treatment responses in clinical trials [44] have also been published. Technical and clinical reviews of serum free light-chain measurement have recently been written by Pratt and Jagannath. [45] [46]
Serum protein electrophoresis or immunofixation is positive in 67-100% of cases, while urine protein electrophoresis or immunofixation is positive in 50-100% of cases. [2] Serum free light chain measurement is positive in 100% of cases, evidence that the underlying monoclonal plasma cells secrete free light chains as well as heavy chains.
Once set, light chain class remains fixed for the life of the B lymphocyte. In a healthy individual, the total kappa-to-lambda ratio is roughly 2:1 in serum (measuring intact whole antibodies) or 1:1.5 if measuring free light chains, with a highly divergent ratio indicative of neoplasm. The free light chain ratio ranges from 0.26 to 1.65. [1]
Light chains only (or Bence Jones protein). This may be associated with multiple myeloma or AL amyloidosis. Heavy chains only (also known as "heavy chain disease"); Whole immunoglobulins. If immunoglobulins tend to precipitate within blood vessels with cold, that phenomenon takes the name of cryoglobulinaemia.
Serum protein electrophoresis showing a paraprotein (spike/peak in the gamma zone) in a patient with multiple myeloma.. A myeloma protein is an abnormal antibody (immunoglobulin) or (more often) a fragment thereof, such as an immunoglobulin light chain, that is produced in excess by an abnormal monoclonal proliferation of plasma cells, typically in multiple myeloma or Monoclonal gammopathy of ...
These events occur particularly in cases where blood cryoglobulin levels of monoclonal IgM are high in patients with IgM MGUS, smoldering Waldenström's macroglobulinemia, or Waldenström's macroglobulinemia and in uncommon cases where the levels of monoclonal IgA, IgG, free κ light chains, or free λ light chains are extremely high in ...
Light chain deposition disease can affect any organ. [3] Renal involvement is always present and can be identified by microscopic hematuria and proteinuria.Due to the gradual buildup of light chains from plasma filtration, renal function rapidly declines in the majority of patients with LCDD as either acute tubulointerstitial nephritis or rapidly progressing glomerulonephritis.
More recently, serum free light chain assays have been utilised in a number of published studies which have indicated superiority over the urine tests, particularly for patients producing low levels of monoclonal free light chains, as seen in nonsecretory multiple myeloma [5] [6] [7] and amyloid light chain amyloidosis (AL amyloidosis). [7] [8 ...